Point-of-Care Ultrasound and the Discovery of Pulmonary Arterial Hypertension in a Teenager.

ABSTRACT: We present a case of a teenage patient with new-onset cardiac symptoms discovered to have primary pulmonary arterial hypertension. Point-of-care ultrasound used early in the patient's presentation identified significant right-sided heart dilatation and dysfunction despite the patient's relatively unrevealing physical examination. This article emphasizes the utility of performing focused cardiac ultrasound in pediatric patients early in their presentation. We briefly review focused cardiac ultrasound technique and highlight relevant literature.

Morphology of the patent ductus arteriosus does not preclude successful patent ductus arteriosus stent implantation in high-risk patients undergoing hybrid stage I palliation: recommendations to optimize ductal stent positioning.

OBJECTIVE: To describe patent ductus arteriosus (PDA) stenting regardless of ductal morphology in high risk patients with hypoplast physiology undergoing hybrid stage I palliation (PDA stenting and placement of bilateral pulmonary artery bands). BACKGROUND: Hybrid palliation is an accepted alternative for patients with hypoplastic left heart syndrome. Patients weighing less than 2.5 kg, history of prematurity, intracranial hemorrhage, and chromosomal abnormality belong to a high-risk group who otherwise might not be ideal candidates for traditional surgical repair (Norwood Operation). METHODS: Between May 2005 and February 2013, a series of 13 high-risk patients with hypoplast physiology with varying types of ductal morphology underwent PDA stenting as part of hybrid stage I palliation. RESULTS: Three major types of ductal morphology were identified: (1) short and semi horizontal, (2) long and semi horizontal, and (3) tortuous. All patients underwent successful PDA stenting. One patient developed proximal coarctation from inadequate coverage of the pulmonary end of the PDA and was successfully treated with a balloon expandable stent 69 days after the initial procedure. CONCLUSIONS: Mid- to long-term follow-up indicates that PDA stents remain widely patent regardless of ductal morphology until comprehensive stage II repair.

Right pulmonary artery thrombosis after hybrid stage 1 palliation: resolution after direct tissue plasminogen activator infusion.

A patient with unbalanced right ventricular dominant atrioventricular septal defect with ascending and transverse arch hypoplasia underwent hybrid stage 1 palliation. On postoperative day 7, he experienced irritability and acute cyanosis. Echocardiography demonstrated a thrombus occluding the right pulmonary artery band. Emergency cardiac catheterization confirmed thrombosis of the right pulmonary artery at the site of the band. Direct infusion of tissue plasminogen activator successfully lysed the thrombus.

Possible association between maternal lithium therapy and premature closure of the arterial duct. A case report.

BACKGROUND: Frequent causes of premature ductal closure include spontaneous idiopathic closure in utero and maternal use of nonsteroidal anti-inflammatory drugs late in pregnancy. CASE: We describe a case of a preterm infant born to a mother treated with lithium throughout pregnancy who presented with right-sided cardiac enlargement at 18 weeks' gestation. Immediately following delivery, echocardiography demonstrated a small closing patent arterial duct. CONCLUSION: We recommend that serial fetal echocardiography with emphasis on Doppler interrogation of the patent arterial duct be performed whenever a pregnant woman is taking lithium. The interrogation of the patent arterial duct is particularly important if right-sided chamber enlargement is noted at fetal sonography as this finding can be an early manifestation of premature ductal constriction.

Unmet Clinical Needs for Transcatheter Pulmonary Valves.

A common feature of congenital heart disease is the presence of right ventricular outflow tract (RVOT) obstruction that can range from mild to severe and can lead to atresia of the pulmonary valve, in extreme conditions. RVOT abnormalities can frequently be corrected surgically or via interventional means. However, most of these patients will ultimately develop pulmonary valve insufficiency and eventual right ventricular dilation, which will require a pulmonary valve replacement at some point in their life to mitigate the detrimental effects of pulmonary valve regurgitation (PVR) on the right ventricle (RV). The evolution from the studies done by Philip Bonhoeffer to implant a pulmonary valve via transcatheter means, have provided a bedrock for transcatheter pulmonary valve replacement (TPVR). Yet, several areas of unmet need for a demographic of patients still exist. Here, we discuss the clinical unmet needs in children under 20 Kg and expand the use of hybrid and other TPVR approaches along with the current indications and contraindications for pulmonary valve replacement. The constraints and limitations from commercially available pulmonary valves will be discussed from a clinical standpoint. Finally, we explore the use of hybrid and periventricular delivery of transcatheter pulmonary valves in younger patients.

Improvement of cardiopulmonary function after minimally invasive surgical repair of pectus excavatum (Nuss procedure) in children.

BACKGROUND: Severe pectus excavatum in children may result in cardiorespiratory functional impairment; therefore, we evaluated cardiopulmonary response to exercise before and after the Nuss procedure. METHODS: Twenty-four physically active pediatric patients aged 9-18 years with severe pectus excavatum (Haller index >3.25) were included in the study. Cardiopulmonary exercise testing using treadmill and modified Bruce protocol was performed before and after the Nuss procedure. RESULTS: Maximal oxygen uptake and oxygen pulse improved by 40.6% (32 ± 13-45 ± 10 ml/kg/min; P = 0.0001) and 44.4% (9 ± 4-13 ± 5 ml/beat; P = 0.03), respectively, after surgical correction of pectus excavatum by Nuss procedure. Significant improvement in maximum voluntary ventilation and minute ventilation after Nuss procedure was also noted. CONCLUSIONS: We found that, after repair of pectus excavatum by Nuss procedure, the exercise capacity as measured by maximal oxygen consumption improved significantly primarily due to increase in oxygen pulse, an indirect measurement of stroke volume.

Ascites secondary to indolent pacemaker infection in a Fontan patient.

This report describes what we believe is the 1st reported case of indolent pacemaker infection's causing abdominal ascites in a patient who has undergone a Fontan operation. Abdominal ascites in a Fontan patient is commonly due to protein-losing enteropathy, systemic venous thrombosis, myocardial dysfunction, chylous ascites, liver cirrhosis, or pancreatitis. Our patient, who had a functional single ventricle, presented with ascites 3 years after undergoing the Fontan operation and pacemaker implantation. After extensive testing and evaluation, we attributed the ascites to indolent infection of the abdominal pacemaker.

Transcatheter ablation of supraventricular tachycardia and closure of a large secundum ASD in a Jehovah's Witness.

This report describes successful radiofrequency ablation of typical atrioventricular (AV) node reentrant tachycardiafollowed by transcatheter closure of a large secundum atrial septal defect in a patient of the Jehovah's Witness faith. Both procedures were performed successfully during the same catheterization, without complication or need for blood transfusion.

Catheter closure of autologous pericardial extracardiac Fontan fenestration.

We describe two patients who required fenestration of an autologous pericardial extracardiac Fontan (APEF), short-medium term angiographic follow-up, and subsequent successful transcatheter closure of the fenestration.

A novel technique to prevent displacement of inferior vena cava filter during cardiac catheterization with subsequent transcatheter closure of a patent foramen ovale in a patient with cryptogenic shock.

We describe a novel technique to prevent displacement of a previously implanted inferior vena cava filter in a patient with cryptogenic stroke who underwent transcatheter closure of a patent foramen ovale. This technique may be of use to patients with cryptogenic stroke who might benefit from transcatheter closure of their patent foramen ovale but would otherwise not be candidates for the procedure because of risk of dislodgement of previously implanted inferior vena cava filter.

Late superior vena cava perforation and aortic laceration after stenting to treat superior vena cava syndrome secondary to fibrosing mediastinitis.

We describe a case of late perforation of the superior vena cava and laceration of the ascending aorta after stent implantation for superior vena cava syndrome. The etiology of the late perforation is unclear, and could be secondary to either flaring of the trailing edge of the stent or chest trauma.

Left atrial thrombus formation on a CardioSeal Septal Occlusion device in a patient with elevated factor VIII: resolution with medical therapy.

We describe a case of thrombus formation on the left atrial disc of a CardioSeal Septal Occlusion device 14 days after transcatheter closure of a patent foramen ovale in a patient with a history of multiple cryptogenic strokes. Work-up to rule out the presence of a hypercoagulable state prior to device closure demonstrated an elevated factor VIII level. Medical therapy with heparin and aspirin was initiated, with subsequent clot resolution.

Transcatheter closure of a patent foramen ovale in an adult with hypoxemia after cardiac transplantation.

We describe a case of refractory hypoxemia secondary to a patent foramen ovale immediately after orthotopic heart transplantation in a 60-year-old woman. The patent foramen ovale was successfully closed with a septal occlusion device, with resolution of the hypoxemia. To our knowledge, transcatheter closure of a patent foramen ovale in an adult patient with refractory hypoxemia during the immediate post-transplant period has not previously been reported.

Contraction of the Amplatzer Vascular Plug I and II in pulmonary artery and systemic venous collateral vessels is safe and of no hemodynamic or vascular consequence in short- and mid-term follow-up.

BACKGROUND: The Amplatzer Vascular Plug (AVP) I and AVP II have been used successfully to occlude moderate-large venous collateral vessels (VC) and pulmonary arteriovenous malformations (PAVM) in children and adults with congenital heart disease. PURPOSE: To report our experience in 4 patients who underwent device occlusion of systemic venous collaterals (3 patients) and device closure of pulmonary arteriovenous malformation (1 patient) that developed contraction of the AVP. METHODS: The records of all patients who underwent device occlusion utilizing both AVP I and AVP II between November 2006 and January 2011 were retrospectively reviewed. All available follow-up chest x-rays were reviewed and compared with angiograms obtained post device occlusion. A device 30%-50% larger than the targeted vessel was utilized to occlude the vessel. RESULTS: Four patients were identified with a mean age of 21 years (range, 7 years and 2 months-52 years) and mean weight of 60.6 kg (range, 15.3-131.5 kg). Two patients received AVP I and 2 patients received AVP II. One patient who received the 12 mm AVP I showed moderate contraction. The 3 other patients who received AVP I (6 mm) and AVP II (10 mm, 12 mm) all demonstrated device contraction to the original shape of the device. Mean follow-up time of 24 months (range, 12-40 months) has shown no evidence of hemodynamic or vascular compromise. CONCLUSIONS: Short to mid-term follow-up indicate that contraction of AVP I and AVP II is safe with no evidence of hemodynamic or vascular compromise. Continued long-term follow-up is warranted.

Ventricular septal avulsion and ventricular septal defect after blunt trauma.

Traumatic ventricular septal defect is an uncommon complication of blunt chest trauma. We report a case of ventricular septal avulsion, associated with traumatic ventricular septal defect and the associated management concerns.

Rare case of persistent left superior vena cava to left upper pulmonary vein: pathway for paradoxical embolization and development of transient ischemic attack and subsequent occlusion with an amplatzer vascular plug.

We report the successful transcatheter closure of a large persistent left superior vena cava draining into a left upper pulmonary vein in a patient who suffered 2 transient ischemic attacks after intravenous (IV) injection of saline flush solution into a left arm peripheral IV line utilizing an Amplatzer Vascular Plug.

Transcatheter fenestration of autologous pericardial extracardiac Fontan via the transhepatic approach.

We describe a case of a patient with hypoplastic left heart syndrome (HLHS) who developed protein-losing enteropathy (PLE) following autologous pericardial extracardiac Fontan (APEF) operation with successful resolution of PLE following transcatheter fenestration via the transhepatic approach.

Transcatheter closure of patent foramen ovale after left ventricular assist device implantation: intraoperative decision making.

The presence of a patent foramen ovale in patients with heart failure can be missed during echocardiography despite the use of provocative maneuvers. Implantation of a left ventricular assist device in these patients can result in right-to-left shunting and hypoxemia. We report the use of a percutaneous atrial septal defect closure device as an alternative to surgical closure.

Seven-year clinical experience with the extracardiac pedicled pericardial Fontan operation.

BACKGROUND: Although improved perioperative outcomes with growth potential of the extracardiac pedicled pericardial Fontan (EPPF) operation have been suggested, no advantage has been demonstrated. METHODS: We retrospectively reviewed our institutional experience of 54 consecutive patients undergoing EPPF between June 1996 and August 2003. Clinical and echocardiographic follow-up was obtained yearly with a mean follow-up of 2.8 +/- 2.0 years. RESULTS: There were 29 males, median age 3.3 years (2-6.8). Median cardiopulmonary bypass time was 79 min (39-295). Fibrillatory arrest was used briefly in 9 patients, of which 6 were for fenestration. One Fontan required takedown (1.8%) and there was 1 death (1.8%) from Candida mediastinitis. Median intensive care unit stay, hospital length of stay, and chest tube drainage were 4 days, 12 days, and 8 days, respectively. Arrhythmias occurred in 7 patients. Three (5.6%) of these had preexisting Holter abnormalities requiring permanent pacemaker implantation. Freedom from thromboembolic events, reoperation, and death at 2.8 years after discharge were 96.2%, 98.1%, and 100%, respectively. All patients were New York Heart Association class I-II, with median oxygen saturation of 94 %. Only 5 patients (9.4%) had mild self-restricted activities. Echocardiographic evaluation revealed excellent ventricular function and flow dynamics. CONCLUSIONS: At midterm follow-up this technique yields outcomes as good as the other Fontan techniques and with further follow-up may prove to be superior. However, at this point no clear advantage has been demonstrated. The low rate of complications and potential for growth are appealing features of this procedure.